Ely Bowman, 4, keeps busy as he receives an infusion of a new drug to treat Batten disease for the first time at Children’s Hospital of Orange County in Orange, CA on Friday, August 25, 2017. Before CHOC offered the treatment, he and his mom had to fly to Ohio every 10 days to receive it. His older brother Titus, 6, died of the disease last September. (Photo by Kevin Sullivan, Orange County Register/SCNG)

Ten days after their 6-year-old son, Titus, died of a rare genetic disease last year, Daniel and Bekah Bowman boarded a plane to begin the grueling fight to save his younger brother.

For the next six months, the Bowmans traveled every 10 days from Irvine to a Columbus, Ohio, children’s hospital where Ely, 4, received an experimental brain infusion for Batten disease. The inherited, fatal disorder, which claimed Titus’ life, eventually leaves children blind, bedridden and demented.

The three-hour time difference and change from sunshine to snow were not unlike the family’s fluid, ongoing passage between hope and grief. The trip to Ohio represented the promise of helping Ely while the trip back meant the emptiness of a home without Titus.

“All the way through, I felt broken but not completely destroyed,” Bekah said. “I learned how to slow down and appreciate each moment and really see the gifts God has given me in those really simple moments. I learned a lot about posturing my heart from a place of gratitude instead of bitterness.”

Bekah Bowman comforts her son Ely, 4, as he receives an infusion of a new drug to treat Batten disease for the first time at Children’s Hospital of Orange County in Orange, CA on Friday, August 25, 2017. Before CHOC offered the treatment, he and his mom had to fly to Ohio every 10 days to receive it. (Photo by Kevin Sullivan, Orange County Register/SCNG)

Bekah and Daniel Bowman keep an eye on their son Ely, 4, as he receives an infusion of a new drug to treat Batten disease for the first time at Children’s Hospital of Orange County in Orange, CA on Friday, August 25, 2017. At right is Dr. Raymond Wang, a metabolic genetics physician, who’s treating the rare and deadly neurodegenerative disorder. Before CHOC offered the treatment, Ely and his mom had to fly to Ohio every 10 days to receive it. (Photo by Kevin Sullivan, Orange County Register/SCNG)

Ely Bowman, 4, keeps busy as he receives an infusion of a new drug to treat Batten disease for the first time at Children’s Hospital of Orange County in Orange, CA on Friday, August 25, 2017. Before CHOC offered the treatment, he and his mom had to fly to Ohio every 10 days to receive it. His older brother Titus, 6, died of the disease last September. (Photo by Kevin Sullivan, Orange County Register/SCNG)

Bekah Bowman smiles as she holds her son Ely, 4, next to her husband Daniel, left, as he receives an infusion of a new drug to treat Batten disease for the first time at Children’s Hospital of Orange County in Orange, CA on Friday, August 25, 2017. Before CHOC offered the treatment, he and his mom had to fly to Ohio every 10 days to receive it. (Photo by Kevin Sullivan, Orange County Register/SCNG)

Dr. Raymond Wang, a metabolic genetics physician at CHOC, talks about treating Batten disease in Ely Bowman, 4, outside his room at Children’s Hospital of Orange County in Orange, CA on Friday, August 25, 2017. Batten disease is a rare and deadly neurodegenerative disorder that’s being treated for the first time on Orange County. (Photo by Kevin Sullivan, Orange County Register/SCNG)

Nurse Melissa Rodriguez, left, prepares to remove Ely Bowman’s infusion as mom Bekah helps distract him at Children’s Hospital of Orange County in Orange, CA on Friday, August 25, 2017. Ely received the treatment for the first time in Orange County after flying to Ohio every 10 days to receive it before. (Photo by Kevin Sullivan, Orange County Register/SCNG)

Bekah Bowman holds her son Ely, 4, after receiving an infusion of a new drug to treat Batten disease for the first time at Children’s Hospital of Orange County in Orange, CA on Friday, August 25, 2017. The infusion is administered through the skull and takes about 4 hours every two weeks. (Photo by Kevin Sullivan, Orange County Register/SCNG)

Bekah Bowman comforts her son Ely, 4, as he receives an infusion of a new drug to treat Batten disease for the first time at Children’s Hospital of Orange County in Orange, CA on Friday, August 25, 2017. Before CHOC offered the treatment, he and his mom had to fly to Ohio every 10 days to receive it. (Photo by Kevin Sullivan, Orange County Register/SCNG)

Bekah Bowman comforts her son Ely, 4, next to her husband Daniel, as he receives an infusion of a new drug to treat Batten disease for the first time at Children’s Hospital of Orange County in Orange, CA on Friday, August 25, 2017. Before CHOC offered the treatment, he and his mom had to fly to Ohio every 10 days to receive it. (Photo by Kevin Sullivan, Orange County Register/SCNG)

Ely Bowman, 4, receives an infusion of a new drug to treat Batten disease for the first time at Children’s Hospital of Orange County in Orange, CA on Friday, August 25, 2017) (Photo by Kevin Sullivan, Orange County Register/SCNG)

Nurse Melissa Rodriguez removes Ely Bowman’s infusion as mom Bekah helps distract him at Children’s Hospital of Orange County in Orange, CA on Friday, August 25, 2017. Ely received the treatment for the first time in Orange County after flying to Ohio every 10 days to receive it before. (Photo by Kevin Sullivan, Orange County Register/SCNG)

Bekah Bowman comforts her son Ely, 4, as he receives an infusion of a new drug to treat Batten disease for the first time at Children’s Hospital of Orange County in Orange, CA on Friday, August 25, 2017. Before CHOC offered the treatment, he and his mom had to fly to Ohio every 10 days to receive it. (Photo by Kevin Sullivan, Orange County Register/SCNG)

Bekah and Daniel Bowman hold their son Ely, 4, as he receives an infusion of a new drug to treat Batten disease for the first time at Children’s Hospital of Orange County in Orange, CA on Friday, August 25, 2017. Before CHOC offered the treatment, he and his mom had to fly to Ohio every 10 days to receive it. (Photo by Kevin Sullivan, Orange County Register/SCNG)

Ely Bowman, 4, keeps busy as he receives an infusion of a new drug to treat Batten disease for the first time at Children’s Hospital of Orange County in Orange, CA on Friday, August 25, 2017. Before CHOC offered the treatment, he and his mom had to fly to Ohio every 10 days to receive it. His older brother Titus, 6, died of the disease last September. (Photo by Kevin Sullivan, Orange County Register/SCNG)

Earlier this year the Bowmans’ burden lightened when Children’s Hospital of Orange County became one of a handful of hospitals nationally to offer the drug, called Brineura. The family’s travel time for Ely’s treatment went from 10 hours, including a layover, to an 18-minute drive to Orange.

Every other Friday, Ely undergoes a four-hour infusion to deliver the enzyme he’s missing to his brain cells. A needle is inserted into a port underneath his scalp and he must remain really still in bed. Afterward, he stays another four hours for observation, but can move around.

“I think he just knows he has to go to a place where they have to play with his head,” Daniel said. “He doesn’t know anything about the disease.”

Dr. Raymond Wang, a CHOC metabolic geneticist, said the goal is to see a gene therapy developed in Ely’s lifetime. He estimated that fewer than 500 American children are living with Batten disease.

“If we keep him seizure-free and he’s not regressing, then he will definitely outlive Titus,” said Wang, who has treated both brothers. “I think I’m hopeful. I obviously don’t want Ely to lose skills. I want him to be able to grow up and do well.”

Rapidly advancing disease

Titus was a normal 3 1/2-year-old when he began having seizures. It took a year before he was diagnosed with Batten disease. Ely was asymptomatic and almost 2 when he also tested positive for the genetic mutation.

The disorder is recessive, meaning that both Bekah, who works in children’s ministry at Journey Christian Church, and Daniel, who coaches track at Concordia University, carry the defective gene. Each child had a 25 percent chance of inheriting both copies.

“It’s hard watching what makes somebody who they are slowly disappear over time,” Wang said. “That’s the cruelest part of the disease. I think watching Titus go from a rambunctious kiddo when I first met him, playing hide and seek in the doctor’s room, and then the next visit seeing him in a wheelchair was really hard.”

In less than two months, Titus lost his ability to walk, talk and see.

But in time, Ely worked to engage, bringing toys to his brother and snuggling with him on a bean bag.

Ely was already asleep when his brother died at home on Sept. 17, 2016.

“When he got up in the morning, he wasn’t there anymore. We were honest about what happened,” Bekah said. “I don’t know how much he got. He was super frustrated a lot of the following days, very angry, very clingy.”

On Sept. 27, the Bowmans flew to Columbus. A few days later, Ely underwent surgery to have the port implanted and received his first infusion Oct. 7.

A friend from church raised about $40,000 for their travel expenses. Another friend put them in touch with a friend in Ohio who let them stay in her home and borrow her car.

Ely is exuberant and affectionate, even with strangers. He attends preschool, loves the Dumbo ride at Disneyland and delights in playing with toy whales in the bathtub. He also has speech delays and struggles with fine motor skills.

FDA approval stirs emotions

After a lengthy approval process, CHOC launched its own clinical trial of the drug in March, allowing the Bowmans to stop flying out of state. Ely is one of four children receiving the infusions there.

In April, the Food and Drug Administration approved Brineura as the first drug for treating a form of Batten disease known as CLN2. One dose costs $27,000 and the couple’s insurance covers the drug, which is no longer considered experimental.

Bekah cheered and sobbed on the day of the FDA’s decision, as a treatment came in time to benefit one son, but too late for another. The bittersweet moment was reminiscent of when she and Daniel found out Ely had been accepted for treatment in Ohio, even as Titus neared death.

“We always say that’s like the epitome of the pain and joy, experienced together, and trying to figure out how to deal with both of those emotions,” Bekah said.

Her sons have also taught her about living joyfully in the midst of tough circumstances.

“Titus lived big in his six years,” Bekah said. “I think that was kind of his mission, to spread a lot of joy in his six years and this guy has taken over.”

Courtney Perkes has covered the medical beat for the Register since 2005. She was queasy when she watched surgery for the first time (a knee replacement) especially when a drop of blood splattered in her notebook! She loves writing about public health issues as well as the courage and resilience of patients facing illness. Courtney strives to lead a healthy lifestyle that includes yoga and not microwaving plastic. She is a graduate of Northwestern University's Medill School of Journalism.

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